Molla Teshome MD, Habtamu Belete MD Aurora Health Care Internal Medicine Residency Program

Molla Teshome MD, Habtamu Belete MD Aurora Health Care Internal Medicine Residency Program

History 32 year-old male who presented with a 4 days history of: Productive cough Right sided pleuritic chest pain Low grade intermittent fever No preceding URTI symptoms, sick contacts or recent travel Hospitalized twice for pneumonia in the past two years Told to have a lung cyst during second admission but declined surgery and biopsy

ROS Intermittent headache, fatigue and decreased appetite Otherwise negative PMH Seizure disorder since childhood Medications Dilantin Allergies NKDA

Family Hx Diabetes in mother Social Hx Smokes ½ PPD for 12yrs, drinks socially, uses marijuana, unemployed

VS: T 100.6, P 108, BP 125/78, RR 22, pulse ox 97% on RA GENERAL: Overweight young male in moderate distress PULM: IC retraction; dullness, decreased air entry and scattered ronchi over lower 1/3 rd of the right lung field posteriorly & laterally CV: Tachycardic, regular rhythm, systolic flow murmur LYMPHATIC: No significant lymphadenopathy The rest of the exam was normal

137 103 15 4.1 25 1.1 ESR - 18 Quantiferon gold negative Respiratory culture No viruses / Bacteria Few Candida Albicans No mycobacteria Blood Culture - negative 13.6 98 19.4 254 39 Bands 6 Neutrophils 79 Lymphocytes 15 Eosinophils 2 Monocytes 3 Basophills 0

Initiated on CAP protocol Failure to improve after 48 hrs of treatment Chest CT and 3D CT with reconstruction was performed

Rare congenital thoracic malformation - representing 0.15 to 6.4 % of all pulmonary malformations Cystic/solid mass composed of nonfunctioning primitive tissue that does not communicate with the tracheobronchial tree. Blood supply is from systemic circulation Two forms Intrapulmonary & Extrapulmonary

Intrapulmonary Extrapulmonary Prevalence 75-90% 10-25% Own Pleural investment None Yes Location 70% on left side >95% on left side Venous drainage Pulmonary veins Systemic veins Fore gut comm. Very rare More common Associated anomalies Uncommon More common

Developmental abnormality in the primitive foregut and lung buds-accessory lung bud Portion of the developing lung mechanically separated from the rest of the organ by compression from CV structures Traction by aberrant systemic vessels Inadequate pulmonary blood flow. Intrapulmonary sequestration may be acquired

Extrapulmonary Commonly diagnosed incidentally May present early with respiratory distress & chronic cough May manifest as GI symptoms and feeding difficulties

Intrapulmonary Usually diagnosed later in childhood or adolescence Commonly presents as recurrent infection Rarely hemoptysis / hemothorax Overdistension may lead to impairment of cardio respiratory function

Primary lung abscess Recurrent pneumonia due to other causes Congenital lung mass Congenital diaphragmatic hernia Bronchogenic cyst Congenital lobar emphysema Chronic lung infection (TB, Fungal.)

Asymptomatic - controversial Resection advocated because of: Likelihood of recurrent infection Need for larger resection if sequestration becomes chronically infected Possibility of hemorrhage from AV anastomoses Symptomatic disease - surgical resection Excision for EPS lesions Lobectomy for IPS lesions

Recurrent infection Heart failure Massive bleeding Fibrous mesothelioma, carcinoma

Antibiotic coverage was broadened Underwent thoracotomy and right lower lobe lobectomy Smooth post op course and discharge

BPS is a rare malformation Intrapulmonary sequestration accounts for more than 75% of cases Possibility of BPS and other congenital malformations should be kept in mind in patients presenting with recurrent infection Treatment is surgery

Landing BH, Dixon LG. Congential malformation and genetic disorders of the respiratory tract (larynx, trachea, bronchi and lungs). AM Rev Respir Dis 1979; 120:151 Van Raemdonck D, De Boeck K, et al. Pulmonary sequestration: a comparison between pediatric and adult patients. Eur J Cardiothorac Surg 2001; 19:388. Kang M, Khandelwal N, et al. Multidetector CT angiography in pulmonary sequestration. J Comput Assist Tomogr 2006; 30:926. Wang HW, Lu JY, Sun JZ, Xiao Y, Wen B. Massive hemoptysis and hemothorax: a rare but fatal complication of intralobar sequestration Chin Med J (Engl) 2012 Jul;125(14):2638-40. Sato Y, Endo S, et al. A rare case of extralobar sequestration with hemoptysis. J Thorac Cardiovasc Surg 2004, 128:778. Gezer S, Testepe I, et al. Pulmonary sequestration: a single-institutional serious composed of 27 cases. J Thorac Cardiovasc Surg 2007; 133: 955

Thank you!